Antibodies to LRP4 in serum and CSF from amyotrophic lateral sclerosis patients

Tzartos, John; Zisimopoulou, Paraskevi; Rentzos, Michael; Karandreas, Nikolaos; Zouvelou, Vasiliki; Evangelakou, Panagiota; Tsonis, Anastasios; Thomaidis, Thomas; Lauria, Giuseppe; Andreetta, Francesca; Mantegazza, Renato; Tzartos, Socrates J.

doi:10.1016/j.jneuroim.2014.08.070

Cited by 26 publications

(46 citation statements)

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“…There is no evidence of specific antibodies or T cells attacking motor neurons in ALS pathogenesis [38], although this may be a result of the primary focus on the motor neurons in ALS research, with little attention drawn to the neuromuscular junction. This is pointed out in a recent study where LRP4 antibodies were detected in sera of 23% of the 104 ALS patients, in which all 104 patients were AChR antibody negative [44].…”

Section: Discussionmentioning

confidence: 83%

Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap

Gotaas

Skeie

Gilhus

2016

Neuromuscular Disorders

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Section: Discussionmentioning

confidence: 83%

Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap

Gotaas

Skeie

Gilhus

2016

Neuromuscular Disorders

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“…Therefore, some actions of these antibodies probably occur elsewhere in the motor system. LRP4 has been shown to play a role in hippocampal neuronal plasticity and astrocyte adenosine triphosphate release, and LRP4 antibodies are seen in the cerebrospinal fluid of ALS patients . It is likely that agrin and LRP4 antibodies play a role in neuronal function elsewhere in the nervous system.…”

Section: Discussionmentioning

confidence: 99%

Agrin and low-density lipoprotein-related receptor protein 4 antibodies in amyotrophic lateral sclerosis patients

et al. 2016

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Introduction The prevalence and characteristics of Agrin and LRP4 antibody positive amyotrophic lateral sclerosis (ALS) patients were studied. Methods We tested 82 ALS patients and 59 controls for Agrin and LRP4 antibodies using ELISA. Results We found that 13.8% of ALS patients had Agrin antibodies, and 9.8% had LRP4 antibodies. Women ALS patients are twice as likely as men to have antibodies. Agrin-positive ALS patients are younger than Agrin-negative ALS patients. Discussion Antibodies to Agrin and LRP4 are found in ALS patients. It must be determined if these antibodies are pathogenic. Since antibody positive patients have upper as well as lower motor neuron findings, the antibodies’ effects cannot be explained solely by their actions at the neuromuscular junction. Perhaps a breakdown in inter-neuronal signaling might cause ALS. Further research is needed to resolve this question.

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“…28 This finding has implications for the specificity of LRP4 Abs for the diagnosis of dSNMG, particularly given that ALS could be a part of the differential diagnosis for patients presenting with muscle weakness. From a clinical perspective, these studies might raise the question of whether immunomodulatory treatment should be attempted in all patients regardless of LRP4 Abs results.…”

Section: Reduction In Antibody Levels Ameliorates the Diseasementioning

confidence: 95%

“…20,22,28,29 Further research on the prevalence of anti-LRP4 Abs in AChR MG, MuSK MG, dSNMG and other neurological diseases is warranted. However, the reported frequency of seropositive LRP4 Abs in different MG patient populations has varied, possibly due to differences in the ethnicities of the studied populations and laboratory techniques used.…”

Section: Clinical Presentation Of Lrp4 Mgmentioning

confidence: 99%

Lipoprotein receptor‐related protein 4 autoantibodies in myasthenia gravis: Where are we and where are we going?

Nakane

Higuchi

Takamatsu

et al. 2019

Clinical & Exp Neuroim

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Myasthenia gravis (MG) is an autoimmune disorder of the neuromuscular junction that leads to fatigue of skeletal muscles and fluctuating weakness. The pathogenesis of MG is sufficiently diverse to include autoimmune mechanisms. In most patients with MG, autoantibodies against acetylcholine receptor can be detected. In a smaller proportion of patients without antiacetylcholine receptor antibodies, autoantibodies to muscle-specific kinase and low-density lipoprotein receptor-related protein 4 (LRP4) are present. LRP4 is critical for neuromuscular junction formation and maintenance. A multimolecular complex is formed among agrin, LRP4 and muscle-specific kinase that is critical for agrin signaling. The presence of anti-LRP4 antibodies is associated with mild symptoms of MG, while thymomas are rarely observed in association with MG and LRP4 antibodies. We review recent findings on the pathogenesis, clinical features and treatment of LRP4 MG.

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Antibodies to LRP4 in serum and CSF from amyotrophic lateral sclerosis patients

Cited by 26 publications

References 0 publications

Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap

Myasthenia gravis and amyotrophic lateral sclerosis: A pathogenic overlap

Agrin and low-density lipoprotein-related receptor protein 4 antibodies in amyotrophic lateral sclerosis patients

Lipoprotein receptor‐related protein 4 autoantibodies in myasthenia gravis: Where are we and where are we going?

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